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1.
Sci Rep ; 14(1): 4214, 2024 02 20.
Artigo em Inglês | MEDLINE | ID: mdl-38378755

RESUMO

Augmentation cystoplasty (AC) is an effective surgical procedure for patients with neurogenic bladder whenever conservative treatments have failed. The present study aimed to determine the risks of metabolic complications, malignancy, long-term outcomes and histopathologic changes of native bladder and the augmented intestine after AC in children with neurogenic bladder. Pediatric patients < 18 years who underwent AC between 2000 and 2020 were enrolled. Early postoperative complications, long-term outcomes and histopathologic changes in mucosal biopsies of native bladder and the augmented intestine after AC were reviewed. Twenty-two patients with a mean age of 7.6 ± 4.4 years were included. The ileum was used in 19 patients and the sigmoid colon in 3 patients. The length of hospital stay was 14.8 ± 6.8 days. Post-operatively, the urinary continence rate improved from 22.7 to 81.8% (p < 0.001). Hydronephrosis resolved in 17 of 19 patients. Vesicoureteral reflux resolved in 16 (64.0%) of the refluxing ureter units and was downgraded in 7 (28.0%). Grades of hydronephrosis and reflux significantly improved following AC (p < 0.001). The estimated glomerular filtration rate also significantly increased (p = 0.012). Formation of urinary tract stones was the most frequent late complication (in 8 patients, 36.4%). Life-threatening spontaneous bladder perforation occurred in 1 patient. After a mean follow-up of 13.4 ± 5.9 years, there were no cases of mortality, new-onset symptomatic metabolic acidosis, or changes in serum electrolytes. Of the 17 patients who were followed for > 10 years, no cases of malignancy or metaplastic changes were identified in the native bladder or augmented bowel epithelium. AC is a safe and effective procedure with low surgical and metabolic complication rates. In addition, AC provides a satisfactory continence rate and long-term protection of renal function, increases functional capacity, and regresses reflux and hydronephrosis. Individualized surveillance is recommended for the early identification of urolithiasis and metabolic disturbances.


Assuntos
Refluxo Gastroesofágico , Hidronefrose , Neoplasias , Bexiga Urinaria Neurogênica , Humanos , Criança , Pré-Escolar , Bexiga Urinaria Neurogênica/complicações , Bexiga Urinaria Neurogênica/cirurgia , Estudos Retrospectivos , Colo Sigmoide , Complicações Pós-Operatórias/etiologia , Refluxo Gastroesofágico/complicações , Hidronefrose/complicações , Neoplasias/complicações
2.
Artigo em Inglês | MEDLINE | ID: mdl-38261995

RESUMO

CONTEXT: Bone loss and fractures are common and serious complications following hematopoietic stem cell transplantation (HSCT), and identifying risk predictors for fractures in transplant recipients remains challenging. The Taiwan Bone Marrow Donation Center is the largest databank of donors in Asia. However, no population-based studies have yet been conducted in Asia to accurately assess the risk of fractures. OBJECTIVE: The aims of this study were to determine the incidence and risk factors for fractures in HSCT recipients. METHODS: We conducted a retrospective cohort study of patients >18 years who received a HSCT from January 1, 2003 to September 30, 2015 using the Taiwan National Health Insurance Research Database. Fractures following HSCT were identified using ICD-9-CM codes. Cox regression analysis was used to identify risk factors for fractures. RESULTS: A total of 3327 patients underwent a HSCT, of whom 126 (3.8%) had a fracture after HSCT. The cumulative incidence of fractures was 5.3% at 5 years, and 10.8% at 10 years. Multivariate analysis showed that a fracture in the 3 years prior to transplant (HR = 3.79; 95% CI 2.39-6.03) was associated with a higher risk of fractures post HSCT. With a daily dose equivalent of >0.50-3.75 mg, >3.75-15.23 mg and >15.23 mg prednisolone, the risk of fractures increased by 1.70 (95% CI 1.07-2.71), 2.23 (95% CI 1.32-3.76) and 2.93 (95% CI 1.43-6.01) folds, respectively. CONCLUSIONS: Regular screening to monitor bone loss should be initiated early, and counseling about the importance of general preventive measures for bone loss is warranted in HSCT recipients with a prior fracture and mean daily dose of steroids >0.50 mg.

3.
J Chin Med Assoc ; 87(3): 314-319, 2024 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-38224231

RESUMO

BACKGROUND: We aimed to assess the effectiveness of the Nuss procedure for pectus excavatum (PE) and explore the impacts of sex and age on outcomes. METHODS: We retrospectively reviewed 594 consecutive children ≤18 years of age who underwent the thoracoscopy-assisted Nuss technique between January 2006 and July 2019. The severity of pectus deformity was calculated according to the Haller index (HI). The classification of PE and clinical data including complications was analyzed. RESULTS: Of the 594 patients, 456 (76.8%) were boys and 138 (23.2%) were girls. The mean age at surgery was 10.0 ± 5.0 years. The most common types of PE were 1A and 2A2 according to Park classification. Intraoperative and postoperative complication rates were 2/594 (0.3%) and 74/594 (12.5%), respectively. The most common complication was bar displacement. The bar was removed in 414 patients 3.5 ± 0.8 years later. The mean preoperative HI, postoperative HI with bar, and HI after bar removal were 4.2 ± 1.7, 2.4 ± 0.3, and 2.7 ± 0.5, respectively. Compared to the preoperative HI, both the postoperative HI with bar and HI after bar removal were significantly lower ( p < 0.001). For preschool-age children, the preoperative HI was significantly higher ( p = 0.027) and the change in HI significantly improved compared to school-age children ( p = 0.004). Boys and adolescents needed significantly more bars and stabilizers. CONCLUSION: Surgical correction of PE using the Nuss procedure is a safe procedure and improves the HI in children of different ages, even in those younger than 6 years of age.


Assuntos
Tórax em Funil , Masculino , Criança , Pré-Escolar , Feminino , Adolescente , Humanos , Tórax em Funil/cirurgia , Estudos Retrospectivos , Complicações Pós-Operatórias , Período Pós-Operatório , Resultado do Tratamento , Procedimentos Cirúrgicos Minimamente Invasivos/métodos
4.
Clin Epidemiol ; 14: 299-307, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35309102

RESUMO

Purpose: Children with vesicoureteral reflux (VUR) are at an increased risk of recurrent urinary tract infections (UTIs). Early detection and treatment of VUR are important to prevent renal function impairment. Therefore, the aims of this study were to determine the epidemiology of VUR and to identify clinical factors associated with VUR in Taiwanese children with a first documented UTI. Patients and Methods: We conducted this nationwide retrospective study using the Longitudinal Health Insurance Database 2010. Children ≤6 years of age who were admitted and received intravenous antibiotics for a newly diagnosed UTI were included. Multivariate logistic regression analysis was used to identify independent factors associated with VUR. Results: Overall, 388 (10.2%) of the children had VUR. The median (interquartile range) age at diagnosis of VUR was 0.5 (0.3-1.3) years. Among the children with VUR, the age at first UTI and the age at diagnosis of VUR were significant lower in the males than in the females. Age ≤1 year at the first UTI (odds ratio (OR), 1.3; 95% confidence interval (CI): 1.0-1.7), renal agenesis and dysgenesis (OR, 4.1; 95% CI: 1.3-13.1), hydronephrosis (OR, 2.2; 95% CI: 1.7-2.9), duplex collecting system/ectopic kidney/ectopic ureter (OR, 13.0; 95% CI: 8.1-20.8), neuropathic bladder (OR, 4.7; 95% CI: 2.0-11.1) and spina bifida (OR, 5.9; 95% CI: 1.3-27.8) were independent factors for VUR. Conclusion: The children with VUR were more likely to have small kidneys and progression to end-stage renal disease. VUR was common in the children with a UTI and who were ≤1 year of age. Clinicians should arrange ultrasound to diagnose urinary tract anomalies. Infants with urinary tract anomalies, neuropathic bladder and spina bifida should receive further voiding cystourethrography to diagnose VUR early, as this may help to prevent renal damage.

6.
J Pediatr Surg ; 56(7): 1157-1161, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-33840505

RESUMO

PURPOSE: Liver transplantation (LT) for small infants < 6 months old is rare but becoming common as perioperative care improves. In Taiwan, living donor LT (LDLT) has expanded indications but is rarely performed for this age group because of unfavorable outcomes in the literature. We evaluated LDLT outcomes of patients <6 months old. METHODS: We identified infants < 6 months old undergoing LDLT between 2004 and 2019 at our hospital. Variables related to recipients, donors, surgeries, and outcomes were analyzed. RESULTS: Nine patients were identified. Indications for LT were biliary atresia (n = 2), Alagille syndrome (n = 1), protein C deficiency (n = 1), and acute liver failure (n = 5), including two patients with neonatal hemochromatosis, one with herpes simplex hepatitis, one with giant cell hepatitis with autoimmune hemolytic anemia, and one with hemophagocytic lymphohistiocytosis. Median age and weight at LT were 129 days and 4.8 kg, respectively. Graft types included left lateral segment (LLS, n = 4), hyper-reduced LLS (n = 4), and monosegment (n = 1). The median graft-to-recipient weight ratio was 4%. The median follow-up period was 14 months (range, 8 days to 127 months) with two mortalities, and two patients were totally weaned off immunosuppressants. Adjuvant therapies were required for patients with giant cell hepatitis and hemophagocytosis. Preoperative reconstructive imaging for estimating graft thickness facilitated surgical planning. CONCLUSION: Although LDLT is difficult to perform for small infants, outcomes are favorable and mainly dependent on underlying causes in addition to technical innovations.


Assuntos
Atresia Biliar , Transplante de Fígado , Atresia Biliar/cirurgia , Sobrevivência de Enxerto , Humanos , Lactente , Recém-Nascido , Doadores Vivos , Estudos Retrospectivos , Taiwan , Resultado do Tratamento
7.
Kidney Int ; 87(3): 632-9, 2015 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-25252027

RESUMO

The incidence of acute kidney injury (AKI) in critically ill children varies among countries. Here we used claims data from the Taiwanese National Health Insurance program from 2006 to 2010 to investigate the epidemiological features and identify factors that predispose individuals to developing AKI and mortality in critically ill children with AKI. Of 60,338 children in this nationwide cohort, AKI was identified in 850, yielding an average incidence rate of 1.4%. Significant independent risk factors for AKI were the use of extracorporeal membrane oxygenation, mechanical ventilation or vasopressors, intrinsic renal diseases, sepsis, and age more than 1 year. Overall, of the AKI cases, 46.5% were due to sepsis, 36.1% underwent renal replacement therapy, and the mortality rate was 44.2%. Multivariate analysis showed that the use of vasopressors, mechanical ventilation, and hemato-oncological disorders were independent predictors of mortality in AKI patients. Thirty-two of the 474 patients who survived had progression to chronic kidney disease or end-stage renal disease. Thus, although not common, AKI in critically ill children still has a high mortality rate associated with a variety of factors. Long-term close follow-up to prevent progressive chronic kidney disease in survivors of critical illnesses with AKI is mandatory.


Assuntos
Injúria Renal Aguda/epidemiologia , Falência Renal Crônica/epidemiologia , Injúria Renal Aguda/mortalidade , Injúria Renal Aguda/terapia , Adolescente , Fatores Etários , Criança , Pré-Escolar , Estado Terminal , Progressão da Doença , Oxigenação por Membrana Extracorpórea , Feminino , Neoplasias Hematológicas/epidemiologia , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Prognóstico , Terapia de Substituição Renal , Respiração Artificial , Fatores de Risco , Sepse/complicações , Sepse/epidemiologia , Taiwan/epidemiologia , Fatores de Tempo , Vasoconstritores/uso terapêutico
8.
Transplantation ; 98(1): 79-87, 2014 Jul 15.
Artigo em Inglês | MEDLINE | ID: mdl-24879380

RESUMO

BACKGROUND: To date, the outcomes of transplant tourism have not been reported extensively. In addition, data about the accuracy of urine cytology for the detection and the role of the BK virus (BKV) in the carcinogenesis of urothelial carcinoma (UC) after renal transplantation are lacking. METHODS: Three hundred seven patients who received deceased donor kidney transplants between January 2003 and December 2009 were retrospectively studied. The clinical parameters and outcomes between the domestic and tourist groups were compared. We also investigated the risk factors and role of BKV in the carcinogenesis of de novo UC by quantitative real-time polymerase chain reaction. RESULTS: The subjects in the tourist group were older at transplantation and had a shorter dialysis time before transplantation. There were significantly higher incidence rates of BKV viruria, Pneumocystis jiroveci pneumonia, and malignancy in the tourist group. Graft and patient survival were superior in the domestic group. A total of 43 cancers were identified, and the most common type of malignancy was UC (23 patients, 53.5%). The tourist group had a significantly higher incidence of tumors. The sensitivity and specificity of urine cytology for detecting UC were 73.9% and 94.7%, respectively. Independent predictors of UC included female sex, use of Chinese herbal medicine, and transplant tourism. Only two patients (8.7%) with UC had detectable BKV. CONCLUSIONS: Transplant tourism was a risk factor for infection and de novo malignancy. Urothelial carcinoma was the most common malignancy after kidney transplantation. Regular screening for the early detection of UC by urine cytology or periodic sonographic surveys is mandatory, especially for those at high risk.


Assuntos
Carcinoma/epidemiologia , Transplante de Rim/efeitos adversos , Turismo Médico , Infecções por Polyomavirus/epidemiologia , Infecções Tumorais por Vírus/epidemiologia , Neoplasias Urológicas/epidemiologia , Urotélio/patologia , Adulto , Vírus BK/genética , Carcinoma/mortalidade , Carcinoma/patologia , Carcinoma/virologia , DNA Viral/sangue , DNA Viral/urina , Medicamentos de Ervas Chinesas/efeitos adversos , Feminino , Sobrevivência de Enxerto , Humanos , Incidência , Estimativa de Kaplan-Meier , Transplante de Rim/mortalidade , Masculino , Pessoa de Meia-Idade , Infecções por Polyomavirus/mortalidade , Infecções por Polyomavirus/patologia , Infecções por Polyomavirus/virologia , Estudos Retrospectivos , Fatores de Risco , Fatores Sexuais , Taiwan/epidemiologia , Fatores de Tempo , Resultado do Tratamento , Infecções Tumorais por Vírus/mortalidade , Infecções Tumorais por Vírus/patologia , Infecções Tumorais por Vírus/virologia , Neoplasias Urológicas/mortalidade , Neoplasias Urológicas/patologia , Neoplasias Urológicas/virologia , Urotélio/virologia
9.
Clin Transplant ; 28(2): 217-22, 2014 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-24433450

RESUMO

Graft-versus-host disease (GVHD) after pancreas transplantation is a rare but serious complication: All previously reported cases were fatal. We herein report three cases of GVHD after pancreas transplantation with favorable outcomes. Patients with a history of kidney (and pancreas) transplantation subsequently received a pancreas (and kidney) transplantation (i.e., pancreas retransplantation or pancreas after kidney transplantation) and developed acute GVHD. All of them responded to increased immunosuppression (e.g., steroid bolus, antithymocyte globulin) and retained normal graft function. Because the clinical manifestations are non-specific, vigilance is necessary to make an accurate diagnosis. We underscored the importance of a biopsy of involved organs and the clinicopathologic correlation in the early diagnosis of GVHD. Augmented immunosuppression to prevent progression from a self-limited disease to life-threatening pancytopenia or sepsis may be most critical to improve outcome.


Assuntos
Doença Enxerto-Hospedeiro/tratamento farmacológico , Imunossupressores/uso terapêutico , Transplante de Pâncreas/efeitos adversos , Pancreatopatias/cirurgia , Adulto , Seguimentos , Doença Enxerto-Hospedeiro/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Pancreatopatias/complicações , Prognóstico , Ensaios Clínicos Controlados Aleatórios como Assunto , Estudos Retrospectivos
10.
J Surg Res ; 187(1): 36-42, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-24246439

RESUMO

BACKGROUND: In this study, we introduced a newly designed totally implantable device for long-term vascular access in rats and compared its efficacy, related complications, and cost-effectiveness with conventional exteriorized jugular vein catheters. METHODS: Forty adult male Sprague-Dawley rats, weighing 250-300 g, were equally divided into two groups (I and II) and all underwent jugular vein catheterization surgery. The totally implanted device was used in group I and conventional exteriorized catheters were used in group II. The functionality of each catheter was checked every 3 d and evaluation included vascular accessibility, patency, and infection. The weight of the animal and microbial culture from the wound and tube were also monitored. We analyzed the cause of vascular access failure and complications, both mechanical and infectious, and compared related variables. RESULTS: The proportions of 9-d patency and 30-d patency in group I were 90% (18/20) and 75% (15/20), respectively, and in group II 80% (16/20) and 35% (7/20), respectively. There was a statistically significant difference in 30-d patency. The rats in group II were more liable to involve vascular access failure because of catheter dislodgment and had a higher infection rate (P = 0.001). Daily body weight gain was also greater in group I than in group II (2.46 ± 0.59 g/d versus 1.84 ± 0.96 g/d; P = 0.02). CONCLUSIONS: This newly designed and totally implanted device substantially increases the success rate of long-term venous access compared with conventional methods. It reinforces the merits of the subcutaneous port and a tethered swivel system and overall has better performance and reliability. Furthermore, given its low cost and the high level of effectiveness offered, this technology could be a powerful tool to be used in future translational medicine research, especially in cases of long-term intravascular administration.


Assuntos
Cateterismo Periférico/economia , Cateterismo Periférico/instrumentação , Sistemas de Liberação de Medicamentos/economia , Sistemas de Liberação de Medicamentos/instrumentação , Dispositivos de Acesso Vascular/economia , Animais , Antibacterianos/farmacologia , Anticoagulantes/farmacologia , Cateterismo Periférico/efeitos adversos , Cefuroxima/farmacologia , Análise Custo-Benefício , Sistemas de Liberação de Medicamentos/efeitos adversos , Desenho de Equipamento , Glucose/farmacologia , Heparina/farmacologia , Veias Jugulares/diagnóstico por imagem , Masculino , Radiografia , Ratos , Ratos Sprague-Dawley , Dispositivos de Acesso Vascular/efeitos adversos , Grau de Desobstrução Vascular
11.
Cell Transplant ; 22(9): 1667-81, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-23051186

RESUMO

Acute paraquat (PQ) poisoning induces redox cycle and leads to fatal injury of lung. Clinical management is supportive in nature due to lack of effective antidote, and the mortality is very high. Mesenchymal stem cells (MSCs) process the properties of immunomodulation, anti-inflammatory, and antifibrotic effects and oxidative stress resistance. MSC transplantation may theoretically serve as an antidote in PQ intoxication. In this study, we examined the potential therapeutic effects of MSCs in PQ-induced lung injury. The degree of PQ toxicity in the rat type II pneumocyte cell line, L2, and MSCs was evaluated by examining cell viability, ultrastructural changes, and gene expression. L2 cells treated with 0.5 mM PQ were cocultured in the absence or presence of MSCs. For the in vivo study, adult male SD rats were administered an intraperitoneal injection of PQ (24 mg/kg body weight) and were divided into three groups: group I, control; group II, cyclophosphamide and methylprednisolone; group III, MSC transplantation 6 h after PQ exposure. MSCs were relatively resistant to PQ toxicity. Coculture with MSCs significantly inhibited PQ accumulation in L2 cells and upregulated the expression of antioxidative heme oxygenase 1 and metallothionein 1a genes, reversed epithelial-to-mesenchymal transition, and increased the viability of PQ-exposed L2 cells. Treatment with MSCs resulted in a significant reduction in severity of liver and renal function deterioration, alleviated lung injury, and prolonged the life span of rats. Altogether, our results suggest that MSCs possess antidote-like effect through multifactorial protection mechanism. The results of this preclinical study demonstrate that transplantation of MSCs may be a promising therapy and should be further validated clinically.


Assuntos
Lesão Pulmonar/induzido quimicamente , Lesão Pulmonar/cirurgia , Transplante de Células-Tronco Mesenquimais/métodos , Paraquat/toxicidade , Adulto , Animais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estresse Oxidativo/efeitos dos fármacos , Paraquat/intoxicação , Distribuição Aleatória , Ratos , Ratos Sprague-Dawley , Adulto Jovem
12.
J Epidemiol ; 22(6): 517-22, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22971550

RESUMO

BACKGROUND: The incidence of idiopathic nephrotic syndrome (INS) varies among countries, with Asia reporting a higher incidence in comparison with Western countries. We investigated the epidemiologic features of INS and attempted to identify factors that predispose individuals to develop end-stage renal disease (ESRD). METHODS: Claims data from the Taiwanese National Health Insurance program from 1996 to 2008 were used to investigate the epidemiologic features and clinical variables of INS (International Classification of Diseases, Ninth Revision, Clinical Modification code, 581) in children younger than 18 years. RESULTS: We enrolled 4083 children (male-female ratio, 1.91:1). During the 13 years of observation, annual incidence decreased from 9.91 to 3.36 per 100 000 children. Annual number of hospital admissions progressively decreased during the first 3 years after diagnosis. At 3.14 ± 2.77 years after INS onset, ESRD had developed in 145 (3.6%) children. Independent predictors of ESRD included older age at onset, acute renal failure (ARF), hypertensive encephalopathy, and a histologic subtype with focal segmental glomerulosclerosis (FSGS). CONCLUSIONS: Pediatric INS in Taiwan was more frequent in boys. Unlike India, the current incidence of pediatric INS in Taiwan is very similar to that reported in Western studies. Older age at disease onset, ARF, hypertensive encephalopathy, and FSGS on biopsy are important predictors of poor renal outcome.


Assuntos
Falência Renal Crônica/epidemiologia , Síndrome Nefrótica/epidemiologia , Adolescente , Distribuição por Idade , Criança , Pré-Escolar , Bases de Dados Factuais , Feminino , Hospitalização/estatística & dados numéricos , Humanos , Lactente , Masculino , Síndrome Nefrótica/terapia , Fatores de Risco , Taiwan/epidemiologia
13.
J Cell Mol Med ; 16(12): 2935-49, 2012 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-22862802

RESUMO

Mesenchymal stem cells (MSCs) have been shown to improve the outcome of acute renal injury models; but whether MSCs can delay renal failure in chronic kidney disease (CKD) remains unclear. In the present study, the were cultured in media containing various concentrations of basic fibroblast growth factor, epidermal growth factor and ascorbic acid 2-phosphate to investigate whether hepatocyte growth factor (HGF) secretion could be increased by the stimulation of these growth factors. Then, TGF-ß1-treated renal interstitial fibroblast (NRK-49F), renal proximal tubular cells (NRK-52E) and podocytes were co-cultured with conditioned MSCs in the absence or presence of ascorbic acid 2-phosphate to quantify the protective effects of conditioned MSCs on renal cells. Moreover, male Sprague-Dawley rats were treated with 1 × 10(6) conditioned MSCs immediately after 5/6 nephrectomy and every other week through the tail vein for 14 weeks. It was found that basic fibroblast growth factor, epidermal growth factor and ascorbic acid 2-phosphate promoted HGF secretion in MSCs. Besides, conditioned MSCs were found to be protective against TGF-ß1 induced epithelial-to-mesenchymal transition of NRK-52E and activation of NRK-49F cells. Furthermore, conditioned MSCs protected podocytes from TGF-ß1-induced loss of synaptopodin, fibronectin induction, cell death and apoptosis. Rats transplanted with conditioned human MSCs had a significantly increase in creatinine clearance rate, decrease in glomerulosclerosis, interstitial fibrosis and increase in CD4(+)CD25(+)Foxp3(+) regulatory T cells counts in splenocytes. Together, our studies indicated that conditioned MSCs preserve renal function by their anti-fibrotic and anti-inflammatory effects. Transplantation of conditioned MSCs may be useful in treating CKD.


Assuntos
Transição Epitelial-Mesenquimal , Fator de Crescimento de Hepatócito/metabolismo , Transplante de Células-Tronco Mesenquimais , Células-Tronco Mesenquimais/fisiologia , Insuficiência Renal Crônica/fisiopatologia , Insuficiência Renal Crônica/terapia , Animais , Apoptose , Ácido Ascórbico/análogos & derivados , Ácido Ascórbico/metabolismo , Linfócitos T CD4-Positivos/imunologia , Células Cultivadas , Técnicas de Cocultura , Creatinina/metabolismo , Progressão da Doença , Fator de Crescimento Epidérmico/metabolismo , Feminino , Fator 2 de Crescimento de Fibroblastos/metabolismo , Fibronectinas/biossíntese , Fibrose , Glomerulosclerose Segmentar e Focal , Humanos , Rim/citologia , Rim/metabolismo , Túbulos Renais Proximais/citologia , Contagem de Linfócitos , Masculino , Células-Tronco Mesenquimais/metabolismo , Proteínas dos Microfilamentos/deficiência , Pessoa de Meia-Idade , Nefrectomia , Podócitos/citologia , Ratos , Ratos Sprague-Dawley , Insuficiência Renal Crônica/metabolismo , Fator de Crescimento Transformador beta1/metabolismo , Adulto Jovem
14.
Pediatrics ; 130(1): e224-9, 2012 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-22732177

RESUMO

Two sisters, aged 15 and 13 years, had previous epithelioid angiomyolipoma of the kidney and suspected thin basement membrane disease, respectively. They presented with 2 years of gross hematuria and new-onset heavy proteinuria. Extensive investigations failed to find an overt cause of their urinary manifestations. The diagnosis of child abuse in a medical setting was confirmed by DNA short tandem repeats analysis, which are the first documented cases in which factitious hematuria was thus diagnosed. Complex forms of child abuse in a medical setting may require forensic tests such as DNA short tandem repeats analysis for diagnosis.


Assuntos
Hematúria/etiologia , Repetições de Microssatélites , Síndrome de Munchausen Causada por Terceiro/diagnóstico , Análise de Sequência de DNA , Adolescente , Feminino , Humanos
15.
Transplantation ; 93(12): 1238-44, 2012 Jun 27.
Artigo em Inglês | MEDLINE | ID: mdl-22499148

RESUMO

BACKGROUND: Focal segmental glomerulosclerosis (FSGS) is a relatively prevalent glomerular disorder that often progresses to end-stage renal disease. Thirty to 80% of kidney transplant (KT) recipients with FSGS will experience recurrence characterized by proteinuria and podocyte damage. We hypothesized that the degree of podocyte foot process (FP) effacement in postreperfusion transplant biopsies can be used to predict the development of clinical recurrence of FSGS. METHOD: Nineteen pairs of pre- and postreperfusion biopsy specimens were studied. We evaluated the degree of FP effacement in postreperfusion KT biopsies by counting the number of widened FP per capillary loop. Early recurrence of FSGS was defined as development of nephrotic range proteinuria between days 3 and 30 posttransplant. RESULTS: Early recurrence occurred in 7 of 19 grafts (36.8%) at a mean of 4.29±1.89 days. The mean score of FP effacement in postreperfusion allograft biopsies was 0.72±0.31 and 1.35±0.63 in the nonrecurrent and recurrent group, respectively (P=0.039). There was an association between FP effacement and proteinuria (P = 0.04). The FP effacement score predicts early recurrence with a sensitivity of 71.4% and specificity of 91.7%. CONCLUSION: FP effacement can be observed within minutes after reperfusion in renal transplantation of recipients with FSGS that will ultimately develop recurrent FSGS. This suggests a key role for the podocyte injury in the pathogenesis of recurrent FSGS and further supports the presence of circulating factors causing FP effacement. The FP effacement score in the postreperfusion KT biopsy may become a useful predictive test if validated in larger studies.


Assuntos
Glomerulosclerose Segmentar e Focal/patologia , Transplante de Rim/efeitos adversos , Podócitos/patologia , Complicações Pós-Operatórias/patologia , Proteinúria/patologia , Adolescente , Adulto , Idade de Início , Biópsia , Criança , Diagnóstico Precoce , Feminino , Glomerulosclerose Segmentar e Focal/cirurgia , Sobrevivência de Enxerto , Humanos , Masculino , Valor Preditivo dos Testes , Proteinúria/cirurgia , Recidiva , Sensibilidade e Especificidade , Transplante Homólogo , Adulto Jovem
16.
J Chin Med Assoc ; 74(9): 419-22, 2011 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-21962251

RESUMO

Chronic graft-versus-host disease (cGVHD) is one of the most frequent and serious complications of allogeneic hematopoietic stem cell transplantation (HSCT). Nephrotic syndrome (NS) is an uncommon and underrecognized manifestation of cGVHD. We report a patient who developed NS 18 months after allogeneic bone marrow transplantation. The onset of NS was accompanied by active manifestations of cGVHD, and immunosuppressants had not been tapered recently. Renal biopsy revealed membranous nephropathy. The patient failed to improve with three combined immunosuppressants (prednisolone, cyclosporine, and mycophenolate mofetil), but achieved partial remission after intravenous immunoglobulin (IVIG) infusion. Twenty-four months after the diagnosis of NS, the patient was still in hematological remission, with normal serum creatinine level, urinary protein loss of 0.7-1.9 g/day and mild oral mucositis. Our report suggests that NS can be a cGVHD-related immune disorder in HSCT patients. Monitoring of renal parameters, especially proteinuria, is important in cGVHD patients. Our case indicated that post-transplant NS, occurring without history of tapering or following immunosuppressant withdrawal, presents a more severe activity of cGVHD and a relatively severe clinical course. IVIG may modify and control the refractory GVHD-related NS, and can be one of the choices of treatment.


Assuntos
Doença Enxerto-Hospedeiro/complicações , Síndrome Nefrótica/etiologia , Transplante de Medula Óssea , Criança , Doença Crônica , Humanos , Terapia de Imunossupressão , Masculino , Síndrome Nefrótica/terapia , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Transplante Homólogo
17.
Transplantation ; 92(6): 709-15, 2011 Sep 27.
Artigo em Inglês | MEDLINE | ID: mdl-21804443

RESUMO

BACKGROUND: Donor-specific antibodies (DSA) are associated with acute kidney graft rejection, but their role in small bowel/multivisceral allograft remains unclear. We carried out a prospective study to understand the impact of DSA in the setting of intestinal allograft rejection. METHODS: Thirteen patients (15 grafts) were serially evaluated for DSA levels pre- and posttransplant. DSA was determined by Luminex and the results were interpreted as fluorescence intensity (FI), with FI more than 3000 considered positive. RESULTS: The clinical rejection episodes in allografts were significantly associated with the presence of DSA (P=0.041).We obtained 291 biopsy samples from graft ileum and date-matched DSA assay reports. Sixty-three (21.65%) of the biopsies showed acute rejection. The appearance of DSA were preformed (n=5, anti-human leukocyte antigen class II=3, anti-class I and II=2), de novo (n=4, 15.25±4.72 days after transplantation, anti-class II=1, and anti-class I and II=3) and never (n=6). Among the 63 biopsies, 30(47.6%) had significant correlations with positive DSA (kappa=0.30, P<0.001) and manifested severe rejection grade (P=0.009). CONCLUSIONS: In this cohort of small bowel/multivisceral transplantation patients, there was a high incidence of DSA. The presence of DSA should alert the clinical team of a higher risk of rejection, and reduction of the FI is clinically associated with resolution. Serial endoscopy guided biopsies combined with simultaneous DSA measurement in postintestinal transplantation follow-up is an effective means of screening for cellular and humoral-based forms of acute rejection.


Assuntos
Intestino Delgado/patologia , Intestinos/transplante , Transplante/métodos , Adolescente , Adulto , Anticorpos/química , Biópsia , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Rejeição de Enxerto , Antígenos HLA/metabolismo , Humanos , Masculino , Pessoa de Meia-Idade , Transplante Homólogo
18.
Cell Transplant ; 20(2): 245-57, 2011.
Artigo em Inglês | MEDLINE | ID: mdl-20719085

RESUMO

Mesenchymal stem cells (MSCs) have been shown to possess immunomodulatory properties. Systemic lupus erythematosus is an autoimmune disease that results in nephritis and subsequent destruction of renal microstructure. We investigated whether transplantation of human umbilical cord blood-derived MSCs (uMSCs) is useful in alleviating lupus nephritis in a murine model. It was found that uMSCs transplantation significantly delayed the development of proteinuria, decreased anti-dsDNA, alleviated renal injury, and prolonged the life span. There was a trend of decreasing T-helper (Th) 1 cytokines (IFN-γ, IL-2) and proinflammatory cytokines (TNF-α, IL-6, IL-12) and increasing Th2 cytokines (IL-4, IL-10). The in vitro coculture experiments showed that uMSCs only inhibited lymphocytes and splenocytes proliferation but not mesangial cells. Long-term engraftment of uMSCs in the kidney was not observed either. Together, these findings indicated that uMSCs were effective in decreasing renal inflammation and alleviating experimental lupus nephritis by inhibiting lymphocytes, inducing polarization of Th2 cytokines, and inhibition of proinflammatory cytokines production rather than direct engraftment and differentiating into renal tissue. Therapeutic effects demonstrated in this preclinical study support further exploration of the possibility to use uMSCs from mismatched donors in lupus nephritis treatment.


Assuntos
Transplante de Células-Tronco de Sangue do Cordão Umbilical , Nefrite Lúpica/terapia , Células-Tronco Mesenquimais/citologia , Cordão Umbilical/citologia , Animais , Anticorpos Antinucleares/imunologia , Proliferação de Células , Citocinas/metabolismo , Progressão da Doença , Feminino , Humanos , Testes de Função Renal , Longevidade , Nefrite Lúpica/complicações , Nefrite Lúpica/imunologia , Nefrite Lúpica/fisiopatologia , Linfócitos/patologia , Células Mesangiais/patologia , Transplante de Células-Tronco Mesenquimais , Camundongos , Proteinúria/complicações , Proteinúria/fisiopatologia , Esclerose/complicações , Esclerose/patologia , Baço/patologia
19.
J Pediatr Hematol Oncol ; 30(5): 366-8, 2008 May.
Artigo em Inglês | MEDLINE | ID: mdl-18458570

RESUMO

BACKGROUND AND AIMS: Various venous approaches are possible during implanting a totally venous access port. The veins, which are commonly used in pediatric patients are internal and external jugular veins. Comparison of different venous approaches has been discussed in a few reports only. The present study reviews the complication rate, operation time of implanting the port via different veins in children. PATIENTS AND METHODS: From January 2003 to December 2005, 94 venous access ports were implanted in 88 consecutive patients in the Pediatric Surgical section of the Taipei Veterans General Hospital. The patients were classified according to the vein used. Group A (n=45), the external jugular vein access group; and group B (n=43), the internal jugular vein access group. RESULTS: The operation time in group A and B were 38.4+/-11.1 and 57.6+/-20.3 min, respectively. The mean operation time of group A was significantly shorter than that of group B (P<0.001). The overall complication rates in group A and B were 8.5%, and 19.1%, respectively. These differences, however, were not significant (P>0.05). CONCLUSIONS: The external jugular vein approach has a shorter operation time and a lower complication rate than the internal jugular vein approach. This approach may be a method of choice in selected pediatric patients.


Assuntos
Cateteres de Demora , Veias Jugulares , Neoplasias Ósseas/tratamento farmacológico , Neoplasias Encefálicas/tratamento farmacológico , Criança , Neoplasias Oculares/tratamento farmacológico , Feminino , Humanos , Leucemia/tratamento farmacológico , Linfoma/tratamento farmacológico , Masculino , Neuroblastoma/tratamento farmacológico , Osteossarcoma/tratamento farmacológico , Retinoblastoma/tratamento farmacológico , Segurança
20.
J Chin Med Assoc ; 71(5): 259-61, 2008 May.
Artigo em Inglês | MEDLINE | ID: mdl-18490231

RESUMO

We discuss the successful saving of an 18-month-old boy with necrotizing fasciitis of the abdominal wall secondary to colon perforation and peritonitis. The patient underwent emergency surgery with exploratory laparotomy, repeated procedures of debridement, and reconstructive abdominal wall surgery with skin graft. He recovered 6 months after admission. We also address the issue of the closure of large abdominal wall defect and the importance of alimentation in this patient group. We discuss the need to recognize necrotizing fasciitis as a potential complication of intra-abdominal disease, as once necrotizing fasciitis occurs, mortality is more likely. We conclude that recognition and aggressive surgical debridement is mandatory.


Assuntos
Parede Abdominal , Doenças do Colo/complicações , Fasciite Necrosante/etiologia , Perfuração Intestinal/complicações , Desbridamento , Fasciite Necrosante/terapia , Humanos , Lactente , Masculino
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